title: The clinical and histopathological spectrum of IgA-pemphigus--report of two cases.
abstract: Two new cases of neutrophilic bullous disease exhibiting bound and circulating intercellular IgA in vivo in both direct and indirect immunofluorescence (IF), for which the term IgA-pemphigus was recently proposed, are presented. The first case showed a unique constellation of clinical and histopathological features not previously described for IgA-pemphigus, resembling both dermatitis herpetiformis and pemphigus, for which the designation IgA-herpetiform pemphigus seems most appropriate. The second case showed clinical and histopathological features mimicking subcorneal pustular dermatosis for which the previously used term IgA-pemphigus foliaceus seems most appropriate. The previously reported 11 cases showing similar direct IF findings, as well as our two patients, illustrate the main differences between IgA-pemphigus and classic forms of this condition: (a) different clinical manifestations with the absence of Nikolsky's sign; (b) scanty acantholysis; an abundance of neutrophils, with occasional neutrophilic spongiosis; (c) absence of C3 on direct IF; (d) the lower sensitivity of indirect IF and low levels of circulating antibodies; (e) the relatively benign course of the disease; (f) the apparent responsiveness to sulphones in many cases. It is suggested that IgA pemphigus should be considered as an entity separate from, but closely related to, classic pemphigus.
authors.affiliation: Department of Dermatology, Beilinson Medical Center, Petah Tikva, Israel.
authors.forename: E
authors.initials: E
authors.lastname: Hodak
authors.forename: M
authors.initials: M
authors.lastname: David
authors.forename: A
authors.initials: A
authors.lastname: Ingber
authors.forename: A
authors.initials: A
authors.lastname: Rotem
authors.forename: B
authors.initials: B
authors.lastname: Hazaz
authors.forename: O
authors.initials: O
authors.lastname: Shamai-Lubovitz
authors.forename: M
authors.initials: M
authors.lastname: Sandbank
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007070:Immunoglobulin A; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D012867:Skin / Q000378:metabolism / Q000473:pathology
chemical_list: D007070:Immunoglobulin A